TY - JOUR
T1 - Transcriptional activation of the cardiac homeobox gene CSX1/NKX2-5 in a B-cell chronic lymphoproliferative disorder
AU - Su, Xinying
AU - Della-Valle, Véronique
AU - Delabesse, Eric
AU - Azgui, Zahia
AU - Berger, Roland
AU - Merle-Béral, Hélène
AU - Bernard, Olivier A.
AU - Nguyen-Khac, Florence
PY - 2008/7/1
Y1 - 2008/7/1
N2 - Homeobox containing transcription factors are frequently deregulated in human hematologic malignant diseases either indirectly through an abnormality of an upstream factor, or directly through rearrangement of the gene itself. Study of T-cell acute lymphoblastic leukemia identified the related non-clustered homeobox transcription factors, TLX1 and TLX3, as frequently ectopically expressed as a result of chromosomal translocations. We report the deregulation of a non-clustered homeobox gene in a new type of t(5;14)(q35;qll) translocation in a mature peripheral B-cell leukemia. This translocation results in the ectopic expression of the CSX1/NKX2-5 gene on chromosome 5q35 due to its juxtaposition to the TCR δ gene on chromosome 14q11. Expression of the CSX1/NKX2-5 protein conferred enhanced replating potential to transduced murine bone marrow cells. Our study establishes that deregulation of homeobox encoding genes is not restricted to acute leukemic proliferations, but is also observed in chronic malignant diseases.
AB - Homeobox containing transcription factors are frequently deregulated in human hematologic malignant diseases either indirectly through an abnormality of an upstream factor, or directly through rearrangement of the gene itself. Study of T-cell acute lymphoblastic leukemia identified the related non-clustered homeobox transcription factors, TLX1 and TLX3, as frequently ectopically expressed as a result of chromosomal translocations. We report the deregulation of a non-clustered homeobox gene in a new type of t(5;14)(q35;qll) translocation in a mature peripheral B-cell leukemia. This translocation results in the ectopic expression of the CSX1/NKX2-5 gene on chromosome 5q35 due to its juxtaposition to the TCR δ gene on chromosome 14q11. Expression of the CSX1/NKX2-5 protein conferred enhanced replating potential to transduced murine bone marrow cells. Our study establishes that deregulation of homeobox encoding genes is not restricted to acute leukemic proliferations, but is also observed in chronic malignant diseases.
KW - Chromosomal translocation
KW - Chronic lymphocytic leukemia
KW - Homeobox
KW - Transcriptional activation
UR - http://www.scopus.com/inward/record.url?scp=46849085783&partnerID=8YFLogxK
U2 - 10.3324/haematol.12595
DO - 10.3324/haematol.12595
M3 - Article
C2 - 18492690
AN - SCOPUS:46849085783
SN - 0390-6078
VL - 93
SP - 1081
EP - 1085
JO - Haematologica
JF - Haematologica
IS - 7
ER -